What is Malignant Peripheral Nerve Sheath Tumors? Malignant peripheral nerve sheath tumors (MPNST) are a rare cancer that affects the protective tissue surrounding nerves coming out of the spinal cord going to the different parts of the body (peripheral nerves). These nerves are outside the central nervous system. The presence of the tumor can cause a lump in the skin, pain in the area and weakness of movement in the affected part.
What is its prevalence ? MPNST is sometimes found in association with neurofibromatis type 1. The frequency of MPNST alone in the general population is about 1 out of 100,000.
How is it diagnosed? Laboratory examination of tumor biopsy material. Imaging scans can help located the tumor and possible metastases.
Is there any specific gene/pathway that has been identified? No specific gene mutation or type of genetic alteration has been linked to MPNST.
How is it treated? As these tumors can spread (metastasize) to other areas of the body, they may be removed surgically. Radiation therapy is used to decrease the chance of recurrence. Chemotherapy may be used if the tumor cannot be completely removed by surgery.
Are there any clinical trials underway ?
None currently known.
How can RareShare be helpful to patients and families?As a free online disease-specific forum, RareShare can be a focal point where patients, families, researchers and healthcare providers can exchange information on MPNST.
RareShare, MGCI Cancer Society and RGI Chapter